ORPHAN EUROPE (RECORDATI GROUP) RECEIVES 2011 CORPORATE AWARD FROM THE
NATIONAL
ORGANIZATION FOR RARE DISORDERS (NORD) FOR THE DEVELOPMENT OF CARBAGLU®
FOR THE
TREATMENT OF NAGS DEFICIENCY

Recordati announces that Orphan Europe has
received the 2011 Corporate Award from the National Organization for
Rare
Disorders (NORD). Orphan Europe was recognized for making Carbaglu®
(carglumic
acid) tablets available in the U.S. for the treatment of a condition
known as
NAGS deficiency.

The award was presented yesterday, 17 May 2011, during NORD's Annual
Gala in
Washington, DC by Patricia C. Richardson on behalf of NORD. "NORD salutes
Orphan
Europe for its commitment to the rare disease community" says
Patricia
Richardson, guest MC with Frank Sasinowski, Chair of the NORD
Board of
Directors. "We are proud to present this 2011 Corporate Award to the
company".

Carbaglu® is the first specific treatment approved by the
FDA for N-
AcetylGlutamate Synthase (NAGS) deficiency, a rare inherited metabolic
disorder
resulting in high levels of ammonium in blood. NAGS deficiency is a
devastating
disease. Newborns presenting with symptoms caused by this condition
require
immediate medical care effective in lowering the high ammonium levels
back to
normal. Should this fail, the condition can lead to permanent damage
to the
brain or death (Albrecht 1998; Broere 2000, Caldovic 2002, Guffon
1995,
Schubiger 1991). To Orphan Europe, it was unacceptable that a
treatment
available to patients in Europe was not available in the US thus
depriving US
patients of the benefits provided by Carbaglu®. The efforts to gain
approval for
Carbaglu® in the US have been rewarded by the granting of marketing
approval by
the FDA in March 2010.

"We are tremendously honored by NORD's acknowledgement of our work" says
Marco
Liguori, CEO of Orphan Europe. "Since our foundation, Orphan Europe has
strived
to make the lives of patients with rare diseases better by
developing and
bringing to market treatments for various rare diseases in Europe. It is
with
great pride and gratefulness that we today receive this award in honor
of our
efforts to expand our mission into the US market."

About Orphan Europe

Orphan Europe was founded in 1990 with the aim to provide treatment for
patients
with unmet medical needs suffering from rare diseases. Since then it has
grown
its business very successfully, and is part of the Recordati Group since
2007.
Orphan Europe provides nine orphan products to patients with the help of
130
medical, scientific and marketing specialists in Europe and Middle
East
countries. In the US, Orphan Europe is present with Carbaglu® for the
treatment
of N-AcetylGlutamate Synthase (NAGS) deficiency. Through an extensive
network of
partnerships the drugs of Orphan Europe are also reaching patients in the
rest
of the world.

Orphan Europe has developed excellent relationships with
clinicians,
researchers, patient groups, health authorities and partnering companies.
Orphan
Europe, which has expertise in gaining European marketing approvals of
orphan
drugs, adapted its infrastructure to the particular requirements of very
small
production batches, distribution network of medicines for rare diseases.
www.orphan-europe.com

About Carbaglu®

Carbaglu® contains carglumic acid, a structural analogue of N-
acetylglutamate.
The biochemical and pharmacological characteristics of Carbaglu®
make it the
specific treatment for NAGS deficiency (Caldovic 2004). It is the only
currently
available product indicated by the US FDA as adjunctive therapy
for the
treatment of acute hyperammonemia due to NAGS deficiency, and as
maintenance
therapy for the treatment of chronic hyperammonemia due to NAGS
deficiency.
Given orally, Carbaglu® stimulates the first enzyme of the urea
cycle, called
CPS 1, triggering the urea cycle and normalizing blood ammonia
concentration
(Tuchman 2008). Uncontrolled hyperammonemia can rapidly result in
brain
injury/damage or death, and prompt use of all therapies necessary to
reduce
plasma ammonia levels is essential.

More information can be found athttp://www.carbaglu.net

NAGS deficiency

NAGS deficiency is an inherited metabolic disorder and it's the rarest
among the
Urea Cycle Disorders (UCDs) (Caldovic 2010). The urea cycle is an
essential
protein metabolic pathway.

NAGS is a mitochondrial liver enzyme that is essential for the urea cycle
(Roth
2006). NAGS catalyzes the formation of N-acetylglutamate (NAG), which then
acts
as an activator of the first enzyme of the urea cycle, CPS 1. If
NAGS is
defective, NAG synthesis is impaired and there is no activation of CPS
1 to
trigger the urea cycle. The activity of the urea cycle is regulated by the
rate
of synthesis of NAG.

The impairment of ammonia detoxification due to NAGS deficiency results in
acute
and chronic hyperammonemia, hyperglutaminemia and,
eventually,
hypocitrullinemia. Hyperammonemia and hyperglutaminemia are particularly
toxic
to the central nervous system (CNS) (Albrecht 1998; Broere 2000).

NAGS deficiency represents a serious life-threatening clinical
condition
(Caldovic 2002, Guffon 1995, Schubiger 1991). Patients with complete
NAGS
deficiency present with acute severe hyperammonemia within the first few
days of
life (Caldovic 2005, Nordenström 2007). The clinical course in
the neonatal
period may be lethal. Left untreated or insufficiently corrected, this
condition
leads to cerebral edema, coma, and eventually death. For those
children who
survive, psychomotor retardation is a frequent outcome (Schubiger
1991).
Patients with partial NAGS deficiency (late-onset) can present
symptoms at
almost any time of life due to any stressful event such as an infection,
trauma,
vaccination (Kingsley 2006), surgery, pregnancy, etc.

-- Adjunctive therapy for the treatment of acute hyperammonemia due
to the
deficiency of the hepatic enzyme N-acetylglutamate synthase (NAGS).

-- Maintenance therapy for the treatment of chronic hyperammonemia due
to the
deficiency of the hepatic enzyme N-acetylglutamate synthase (NAGS).

Carbaglu® Important Safety Information

Carbaglu® treatment should be initiated by a physician
experienced in the
treatment of metabolic disorders. Any episode of acute
symptomatic
hyperammonemia should be treated as a life-threatening
emergency.Treatment of
hyperammonemia may require dialysis, preferably hemodialysis, to remove a
large
burden of ammonia. Uncontrolled hyperammonemia can rapidly result in
brain
injury/damage or death, and prompt use of all therapies necessary to
reduce
plasma ammonia levels is essential.

Management of hyperammonemia due to NAGS deficiency should be
done in
coordination with medical personnel experienced in metabolic disorders.
Ongoing
monitoring of plasma ammonia levels, neurological status, laboratory
tests and
clinical responses in patients receiving Carbaglu is crucial to assess
patient
response to treatment. Plasma ammonia levels should be maintained within
normal
range for age via individual dose adjustment. During acute
hyperammonemic
episodes, protein restriction and hypercaloric intake is recommended to
block
ammonia generating catabolic pathways. When plasma ammonia levels
have
normalized, protein intake can usually be increased with the
goal of
unrestricted protein intake.

The most common adverse reactions in more than 13% of patients are:
infections,
vomiting, abdominal pain, pyrexia, tonsilitis, anemia, ear infection,
diarrhea,
nasopharyngitis, and headache.

To report SUSPECTED ADVERSE REACTIONS, contact Accredo Health Group Inc. at
1-888-454-8860, or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
No drug interaction studies have been performed with Carbaglu®, and
human milk-
feeding is not recommended.
Carbaglu® tablets should not be swallowed whole or crushed. Each
tablet should
be dispersed in a minimum of 2.5 mL of water. Carbaglu tablets do not
dissolve
completely in water and undissolved particles of the tablet may remain
in the
mixing container. The mixing container should be rinsed with additional
volumes
of water and the contents swallowed immediately. USE IN OTHER FOODS AND
LIQUIDS
HAS NOT BEEN STUDIED CLINICALLY AND IS THEREFORE NOT RECOMMENDED.
Before opening, store refrigerated at 2 to 8 oC (36 to 46 oF). After
first
opening of the container do not refrigerate, and do not store above 30
°C (86
°F). Keep the container tightly closed in order to protect from
moisture. Write
the date of opening on the tablet container. Discard one month after
first
opening.

Recordati, established in 1926, is a European pharmaceutical group,
listed on
the Italian Stock Exchange (Reuters RECI.MI, Bloomberg REC IM,
ISIN IT
0003828271),with a total staff of over 2,800, dedicated to the
research,
development, manufacturing and marketing of pharmaceuticals. It has
headquarters
in Milan, Italy, operations in the main European countries, and a
growing
presence in the new markets of Central and Eastern Europe. A European
field
force of around 1,400 medical representatives promotes a wide
range of
innovative pharmaceuticals, both proprietary and under license, in a
number of
therapeutic areas including a specialized business dedicated to
treatments for
rare diseases. Recordati's current and growing coverage of the
European
pharmaceutical market makes it a partner of choice for new product licenses
from
companies which do not have European marketing organizations.
Recordati is
committed to the research and development of new drug entities
within the
cardiovascular and urogenital therapeutic areas and of treatments for
rare
diseases. Consolidated revenue for 2010 was EUR 728.1 million, operating
income
was EUR 154.8 million and net income was EUR 108.6 million.

Statements contained in this release, other than historical facts, are
"forward-
looking statements" (as such term is defined in the Private
Securities
Litigation Reform Act of 1995). These statements are based on
currently
available information, on current best estimates, and on assumptions
believed to
be reasonable. This information, these estimates and assumptions may prove
to be
incomplete or erroneous, and involve numerous risks and uncertainties,
beyond
the Company's control. Hence, actual results may differ materially from
those
expressed or implied by such forward-looking statements. All
mentions and
descriptions of Recordati products are intended solely as information
on the
general nature of the company's activities and are not intended to
indicate the
advisability of administering any product in any particular instance.

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